Waldenstrom's disease - is extremely rare tumor of the bone marrow, which is formed from mutated plazmotsitarnyh and lymphocytic cells. This pathological condition is accompanied by the appearance of cells that produce excessive amounts of immunoglobulin. Over the violation is similar to chronic lymphocytic leukemia.
The most common pathology manifested symptomatic in older age. This violation is very dangerous, because It carries the risk of serious complications. Macroglobulinemia requires complex treatment.
The causes of the disease
The exact reasons why there is Waldenstrom's macroglobulinemia, not yet installed. There is a genetic therapy of this disease. It is not without reason, that is. To. Is not uncommon to identify the disease in members of one family, related by blood.
In addition, many researchers of this pathological condition tend to virusogeneticheskoy macroglobulinemia theory of this type. It is believed some viruses can be introduced into cells and cause genetic failure. Thus by dividing cells appear with mutated DNA. Often it suffers and immunity, which can not distinguish between infected cells and elements from defect structure. This leads to the appearance of plasma cells and lymphocytes are unable to perform its function and secrete large amounts of protein - immunoglobulin.
Factors that increase the risk of developing this disease condition are:
- contact with radioactive substances;
- taking certain medications;
- effects of heavy metals and other hazardous chemicals;
- prolonged exposure to the sun;
- trauma accompanied with fractures of bones;
- cases of neuropathy in history;
- frequent stay in a state of stress.
It is believed that contribute to the emergence of this disease may be a long stay in ecologically unfavorable areas and bad habits.
symptoms
When Waldenstrom macroglobulinemia symptoms in the early stages are similar to lymphocytic leukemia. Initial symptoms of this cancer are general in nature. The patient will complain to:
- weakness;
- increased sweating;
- rapid weight loss;
- pain and aches in the joints;
- decreased performance;
- subfebrile temperature.
In the future, the emergence of the lymphoid infiltration of the symptoms observed. Is an increase in lymph nodes. Patients are feeling pressure in hypochondria because of an enlarged spleen.
As the progression of the pathological condition in a patient having symptoms of hemorrhagic syndrome. Due to the increase of protein level in blood platelet adhesion processes violated. This increases the risk of bleeding. Very often occur nasal and gingival bleeding. There may be a pale skin. Identified point of bleeding into the skin. Patients become sluggish. Possible fainting. In severe pathological condition of constraint limb mobility is observed due to the dysfunction of the joints.
In the later stages of disease development paraamiloidoza bright signs appear. There is an increase in the size of the language. The patient loses the ability to speak normally. There may be difficulty with swallowing. Protein compounds are deposited on all the internal organs. Particularly strong is suffering from cardio-vascular system. Patients appear tachycardia and other violations of the heart.
Due to the accumulation of proteins in the body may experience pain in different parts of the body. In the later stages of the disease may cause disorders of the kidneys and lungs. Often symptoms occur in the central nervous system.
Diagnostics
To confirm such a pathological condition as Waldenstrom's macroglobulinemia, it requires a comprehensive examination. First, the doctor collects clinical and family history. The presence of pathology indicates a number of deviations that emerge during the general and biochemical blood tests.
The presence of the pathology indicated elevated ESR and matted erythrocytes, as well as increased content lymphocyte cells. They can be identified and immature leukocytes. The blood in this pathology is also detected elevated levels of urea, glucose, cholesterol, electrolytes violation. Often determined by elevated levels of calcium and creatinine. Performed a urine test to detect protein.
To confirm the diagnosis is often administered immunoelectrophoresis. This study reveals high blood immunoglobulin M. trephine biopsy of iliac bone to obtain bone marrow is often administered. After that the study of samples obtained. It can be carried out biopsy tissue, where there are deposits of the protein.
To assess the state of the spleen, liver, and other internal organs assigned US. To detect secondary tumor localization and enlarged lymph nodes assigned holding spiral computer tomography. Often performed to confirm the diagnosis of MRI, allows clarify the status of the internal organs. This study is able to reveal the layering of proteins on the bodies. It can be refined state of cerebral vessels. To evaluate heart function is assigned to the ECG.
disease treatment
Macroglobulinemia Waldenstrom can be treated by various methods. In identifying the disease at early stages of development, the doctor may take a wait and see attitude. With increasing speed of development of the tumor process can be prescribed treatment.
patients undergoing chemotherapy is often recommended. In the treatment of cytostatics used, allowing to destroy the cells with signs of cancerous degeneration. If there are signs of anemia assigned administering RBC.
It may be recommended application extracorporeal techniques. Hemosorption often prescribed procedures, dialysis and plazmafareza. They allow you to remove the body of the pathological protein and reduce the risk of massive bleeding.
In most cases, recovery can only be achieved by bone marrow transplantation. This method of treatment is rarely used because Elderly patients difficult to tolerate this procedure. Transplantation is associated with the risk of complications. To reduce the risks recommended bone marrow is collected from a related donor.
Complications and prognosis of the patient's life expectancy
If not promptly take measures aimed at eliminating such pathological conditions such as Waldenstrom macroglobulinemia, proteins can occlude the vessel lumen and to provoke bleeding.
Increased bleeding may be a cause of severe anemia. Due to blockage of blood vessels in the brain may die off of the body tissue. Perhaps the development paraproteimicheskoy coma. Frequent complications of this disease - renal dysfunction and urinary tract. Often there is the accession of infection. It may impair lung function.
The life expectancy of patients with this diagnosis in complex treatment is 7 years. Full recovery is possible to achieve approximately 30% of patients. Complications worsens the prognosis of a pathological condition.
